This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Familial mediterranean fever

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Familial Mediterranean fever (FMF) is characterized be episodes of fever, peritonitis and/or pleuritis

  • is an autosomal-recessive, hereditary auto-inflammatory disease and has a reference in the Online Mendelian Inheritance in Man database (OMIM) ID: 249100
  • FMF occurs as a result of mutations in the MEditerranean FeVer (MEFV gene)
    • is the only gene currently known to be associated with FMF and is located on chromosome 16
  • primary characteristic of FMF is
    • recurrent fever and serositis
      • which results in pain in the abdomen, chest, joints, muscles, etc.
    • amyloidosis, skin lesions and arthritis may be seen in these patients
      • the most severe complication of FMF is amyloid A (AA) amyloidosis leading to renal failure

This condition occurs most often in non-Ashkenazi Jews, Armenians and Arabs. It is not restricted to these groups and may occur in, among others, people of Ashkenazi Jewish, Italian, and Anglo-saxon descent.

There are mainly two phenotypes in FMF

  • Type 1 is commonly associated with recurrent short episodes of inflammation and serositis, including fever, peritonitis, synovitis, pleuritis, and rarely
    pericarditis and meningitis
    • symptoms and severity vary from one person to another
    • typical clinical manifestations of FMF type 1 usually last from 12 to 72 hours and include the following typical attacks (2):
      • 1) recurrent fever, characterized by a temperature ranging from 38 degrees C to 40 C;
      • 2) abdominal attacks, featuring abdominal pain (usually the entire abdomen is involved);
      • 3. arthritic attacks, frequently featuring as monoarthritis localized in the large joints of the leg (hip, knee, ankle);
      • 4. chest attacks, including pleuritis and pericarditis;
      • 5. pre-attack symptoms, occurring 12 to 24 hours before any FMF attacks, usually including discomfort, abnormal taste sensation,
        dizziness, increased appetite, irritability, etc.
  • Type 2 FMF is characterized by amyloidosis as the first clinical manifestation of the disease, in otherwise asymptomatic people

A systematic review with respect to interventions in familial Mediterranean fever (1):

  • based on limited evidence, review (10 RCTs; n= 312) found three times daily colchicine may reduce number of attacks and outcomes after single dose and divided doses may not be different in children
  • for colchicine-resistant disease, anakinra and canakinumab might be effective

Reference:

  • Yin X, Tian F, Wu B, Xu T. Interventions for reducing inflammation in familial Mediterranean fever. Cochrane Database of Systematic Reviews 2022, Issue 3. Art. No.: CD010893. DOI: 10.1002/14651858.CD010893.pub4
  • Lidar M et al.The prodrome: aprominent yet overlooked preattack manifestation of familial Mediterranean fever. Journal of Rheumatology 2006;33(6):1089-92.

Create an account to add page annotations

Add information to this page that would be handy to have on hand during a consultation, such as a web address or phone number. This information will always be displayed when you visit this page

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

Connect

Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.